Meningocele, Cranial - Frontoethmoidal
Carole Chenier RDMS 01 January 2001
Cephalocele is the protrusion of intracranial contents through a bony defect in the skull. Cranial meningocele refers to herniation of meninges only. Originally considered as one of the varied manifestations of failed neural tube closure such as anencephaly or spina bifida, epidemiologic data suggests that isolated frontoethmoidal cephalocele is a pathogenetically distinct condition. Families with this malformation are not at increased risk for neural tube defect in subsequent offspring, but may present with other central nervous system anomalies (6%). Thailand has an unusually high incidence of frontal cephaloceles.A sac protruding through an anterior cranial defect may be seen on a sagittal or transverse views through the orbital region. Hypertelorism may result.
Although most fronto-ethmoidal cephaloceles are sporadic, occasionally they occur in conjunction with known genetic syndromes, and may present with characteristic associated defects. For example, in Roberts syndrome, an autosomal recessive disorder, there may be associated growth restriction, limb reduction defects, cleft lip and/or palate.
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